Treatment of Epstein Barr virus-induced haemophagocytic lymphohistiocytosis with rituximab-containing chemo-immunotherapeutic regimens. Br J Haematol 2013 Aug;162(3):376-82
Date
05/23/2013Pubmed ID
23692048Pubmed Central ID
PMC3776423DOI
10.1111/bjh.12386Scopus ID
2-s2.0-84880319443 (requires institutional sign-in at Scopus site) 173 CitationsAbstract
Haemophagocytic lymphohistiocytosis (HLH) is a life threatening complication of Epstein-Barr virus (EBV) infection. The anti-CD20 antibody rituximab depletes B cells, leading to improved outcomes for patients with EBV-associated B-lymphoproliferative disorders. To gather data on the use of rituximab in EBV-HLH, we performed a retrospective investigation involving 42 EBV-HLH patients who had received treatment with rituximab-containing regimens. On average, patients received 3 rituximab infusions (range 1-10) at a median dose of 375 mg/m(2) . In all patients, rituximab was administered with other HLH-directed medications, including steroids, etoposide and/or ciclosporin. Rituximab-containing regimens appeared well tolerated and improved clinical status in 43% of patients. Examination of laboratory data obtained prior to and within 2-4 weeks after the first rituximab dose revealed significant reductions in EBV load (median load pre-rituximab: 114,200 copies/ml, median post-rituximab: 225 copies/ml, P = 0.0001) and serum ferritin levels (median ferritin pre-rituximab: 4260 μg/l, median post-rituximab: 1149 μg/l, P = 0.001). Thus, when combined with conventional HLH-directed therapies, rituximab improves symptoms, reduces viral load and diminishes inflammation. These data support the incorporation of rituximab into future prospective clinical trials for patients with EBV-HLH.
Author List
Chellapandian D, Das R, Zelley K, Wiener SJ, Zhao H, Teachey DT, Nichols KE, EBV-HLH Rituximab Study GroupAuthor
Julie-An M. Talano MD Professor in the Pediatrics department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
AdolescentAdult
Aged
Antibodies, Monoclonal, Murine-Derived
Antiviral Agents
Child
Child, Preschool
Drug Administration Schedule
Drug Evaluation
Drug Therapy, Combination
Epstein-Barr Virus Infections
Female
Herpesvirus 4, Human
Humans
Immunologic Factors
Lymphohistiocytosis, Hemophagocytic
Male
Middle Aged
Prognosis
Retrospective Studies
Rituximab
Survival Analysis
Treatment Outcome
Viral Load
Young Adult
